Hypoplastic left heart syndrome

Description

A spectrum of congenital cardiovascular malformations with normally aligned great arteries without a common atrioventricular junction, characterised by underdevelopment of the left heart with significant hypoplasia of the left ventricle including atresia, stenosis, or hypoplasia of the aortic or mitral valve, or both valves, and hypoplasia of the ascending aorta and aortic arch.

Index terms

Hypoplastic left heart syndrome
HLHS - [hypoplastic left heart syndrome]
aortic valve atresia, in hypoplastic left heart syndrome

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ascending aorta hypoplasia, in hypoplastic left heart syndrome
atresia of mitral valve, in hypoplastic left heart syndrome
atresia or marked hypoplasia of aortic orifice or valve, with hypoplasia of ascending aorta and defective development of left ventricle
atresia or marked hypoplasia, of aortic orifice or valve, with hypoplasia of ascending aorta and defective development of left ventricle with mitral valve atresia
hypoplasia of aortic valve, in hypoplastic left heart syndrome
hypoplasia of the left heart
left heart hypoplasia syndrome

Exclusions from above levels

http://id.who.int/icd/entity/733825440 – Inborn errors of metabolism

Sibling codes

LA89.0 – Double inlet atrioventricular connection
LA89.1 – Tricuspid atresia
LA89.2 – Mitral atresia
LA89.Y – Other specified functionally univentricular heart
LA89.Z – Functionally univentricular heart, unspecified

Foundation entity

Hypoplastic left heart syndrome – http://id.who.int/icd/entity/1811800027

Part of: LA89: Functionally univentricular heart › 20: Developmental anomalies